Anti-phospholipase A2 receptor in Non-lupus Patients with Membranous Nephropathy and Crescents

Introduction: Anti-phospholipase A2 receptor (PLA2R) is detected in approximately 70% of biopsies of “primary” membranous nephropathy (MN). Crescents in MN in non-lupus patients suggest additional injury, such as antineutrophil cytoplasmic antibody (ANCA) or anti-glomerular basement membrane (anti-...

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Autores principales:	Yiqin Zuo, Livia Barreira Cavalcante, James Monroe Smelser, Neil Sanghani, Jamie P. Dwyer, Julia Breyer Lewis, Agnes B. Fogo
Formato:	article
Lenguaje:	EN
Publicado:	Karger Publishers 2021
Materias:	Diseases of the endocrine glands. Clinical endocrinology RC648-665
Acceso en línea:	https://doaj.org/article/f5b1bbcb1dac419d990eb32fb9487b75
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spelling	oai:doaj.org-article:f5b1bbcb1dac419d990eb32fb9487b752021-12-02T12:40:24ZAnti-phospholipase A2 receptor in Non-lupus Patients with Membranous Nephropathy and Crescents2673-363310.1159/000520641https://doaj.org/article/f5b1bbcb1dac419d990eb32fb9487b752021-11-01T00:00:00Zhttps://www.karger.com/Article/FullText/520641https://doaj.org/toc/2673-3633Introduction: Anti-phospholipase A2 receptor (PLA2R) is detected in approximately 70% of biopsies of “primary” membranous nephropathy (MN). Crescents in MN in non-lupus patients suggest additional injury, such as antineutrophil cytoplasmic antibody (ANCA) or anti-glomerular basement membrane (anti-GBM)-associated glomerulonephritis and is postulated to reflect injury by a mechanism that unmasks cryptic epitopes leading to the second autoantibody. Methods: We studied PLA2R staining in non-lupus patients with MN and crescents. Native renal biopsies in 16 non-lupus patients with MN and crescents were stained for PLA2R. Results: The patients included 5 women and 11 men, with mean age 61 yrs and elevated serum creatinine (mean 4.68 mg/dL). Hematuria and proteinuria (mean 4.97 g/d) were documented in 13 patients. Two patients had positive serum anti-GBM antibody. Nine of eleven patients tested for ANCA were positive, with p-ANCA (n=4), c-ANCA (n=2), or both (n=1), with two not specified. On average, 27% of glomeruli had crescents. One patient had an initial biopsy with MN, 4 years later had MN with crescent, and 7 years later had rebiopsy with persistent MN with crescents. One patient had ANCA-associated vasculitis, and 5 years later had MN and crescent. The remaining 14 patients had concurrent diagnoses of MN and crescents. PLA2R was positive in 5 cases, 3 with ANCA positivity, 2 with unknown ANCA status, and none with anti-GBM disease. The patient with initial MN preceding crescent was PLA2R positive; the patient with initial ANCA-associated vasculitis preceding MN was PLA2R negative. Conclusions: Most patients (64%) presented with concomitant MN and crescents, with rare occurrence of an initial disease process followed later by the second injury. PLA2R was positive in 31% of patients, suggesting most are secondary MN. Further study to determine the cryptic epitopes may shed light on the triggering mechanisms for these rare but unlikely coincidental glomerular injuries.Yiqin ZuoLivia Barreira CavalcanteJames Monroe SmelserNeil SanghaniJamie P. DwyerJulia Breyer LewisAgnes B. FogoKarger PublishersarticleDiseases of the endocrine glands. Clinical endocrinologyRC648-665ENGlomerular Diseases (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Diseases of the endocrine glands. Clinical endocrinology RC648-665
spellingShingle	Diseases of the endocrine glands. Clinical endocrinology RC648-665 Yiqin Zuo Livia Barreira Cavalcante James Monroe Smelser Neil Sanghani Jamie P. Dwyer Julia Breyer Lewis Agnes B. Fogo Anti-phospholipase A2 receptor in Non-lupus Patients with Membranous Nephropathy and Crescents
description	Introduction: Anti-phospholipase A2 receptor (PLA2R) is detected in approximately 70% of biopsies of “primary” membranous nephropathy (MN). Crescents in MN in non-lupus patients suggest additional injury, such as antineutrophil cytoplasmic antibody (ANCA) or anti-glomerular basement membrane (anti-GBM)-associated glomerulonephritis and is postulated to reflect injury by a mechanism that unmasks cryptic epitopes leading to the second autoantibody. Methods: We studied PLA2R staining in non-lupus patients with MN and crescents. Native renal biopsies in 16 non-lupus patients with MN and crescents were stained for PLA2R. Results: The patients included 5 women and 11 men, with mean age 61 yrs and elevated serum creatinine (mean 4.68 mg/dL). Hematuria and proteinuria (mean 4.97 g/d) were documented in 13 patients. Two patients had positive serum anti-GBM antibody. Nine of eleven patients tested for ANCA were positive, with p-ANCA (n=4), c-ANCA (n=2), or both (n=1), with two not specified. On average, 27% of glomeruli had crescents. One patient had an initial biopsy with MN, 4 years later had MN with crescent, and 7 years later had rebiopsy with persistent MN with crescents. One patient had ANCA-associated vasculitis, and 5 years later had MN and crescent. The remaining 14 patients had concurrent diagnoses of MN and crescents. PLA2R was positive in 5 cases, 3 with ANCA positivity, 2 with unknown ANCA status, and none with anti-GBM disease. The patient with initial MN preceding crescent was PLA2R positive; the patient with initial ANCA-associated vasculitis preceding MN was PLA2R negative. Conclusions: Most patients (64%) presented with concomitant MN and crescents, with rare occurrence of an initial disease process followed later by the second injury. PLA2R was positive in 31% of patients, suggesting most are secondary MN. Further study to determine the cryptic epitopes may shed light on the triggering mechanisms for these rare but unlikely coincidental glomerular injuries.
format	article
author	Yiqin Zuo Livia Barreira Cavalcante James Monroe Smelser Neil Sanghani Jamie P. Dwyer Julia Breyer Lewis Agnes B. Fogo
author_facet	Yiqin Zuo Livia Barreira Cavalcante James Monroe Smelser Neil Sanghani Jamie P. Dwyer Julia Breyer Lewis Agnes B. Fogo
author_sort	Yiqin Zuo
title	Anti-phospholipase A2 receptor in Non-lupus Patients with Membranous Nephropathy and Crescents
title_short	Anti-phospholipase A2 receptor in Non-lupus Patients with Membranous Nephropathy and Crescents
title_full	Anti-phospholipase A2 receptor in Non-lupus Patients with Membranous Nephropathy and Crescents
title_fullStr	Anti-phospholipase A2 receptor in Non-lupus Patients with Membranous Nephropathy and Crescents
title_full_unstemmed	Anti-phospholipase A2 receptor in Non-lupus Patients with Membranous Nephropathy and Crescents
title_sort	anti-phospholipase a2 receptor in non-lupus patients with membranous nephropathy and crescents
publisher	Karger Publishers
publishDate	2021
url	https://doaj.org/article/f5b1bbcb1dac419d990eb32fb9487b75
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Anti-phospholipase A2 receptor in Non-lupus Patients with Membranous Nephropathy and Crescents

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